软组织内腱鞘巨细胞瘤的诊治:附33例病例分析

Diagnosis and Treatment of Extra-articular Tenosynovial Giant Cell Tumor in Soft Tissue:A Report of 33 Cases

目的探讨罕见部位关节外型腱鞘滑膜巨细胞瘤(tenosynovial giant cell tumor,TSGCT)的临床特征、影像学表现及预后,以提高对该病的认识。方法检索文献中报道的病例,结合本院收治的2例患者资料进行回顾性分析。结果33例患者纳入本次研究,其中男性17例,女性16例,平均年龄39.8岁,肿瘤最大直径平均值为7.2 cm。发病部位多为大腿、臀部及头颈部。主要的临床表现为单纯包块(39.4%)和疼痛伴肿胀(12.1%)。CT检查表现为等密度或稍低密度的软组织肿块,病灶内无钙化或骨化。MRI主要表现为T1WI低信号或等-低信号,T2WI大部分(95.2%)为混杂信号,增强多为不均匀强化。17例获得随访的患者术后发生复发或转移的风险为17.6%。结论完全关节外型TSGCT非常罕见。对于来源于下肢及头颈部、MRI表现为混杂信号、CT显示关节外来源的软组织肿块需要考虑到该病,手术能有效控制其进展。

Objective To investigate the clinical features,imaging manifestations and prognosis of tenosynovial giant cell tumor(TSGCT)in a rare site in order to improve the understanding of the disease.Methods Thirty-one cases reported in the literature were retrieved,and the data of two patients admitted to our hospital were retrospectively analyzed.Results Among the 33 patients,17 were male and 16 were female,with a mean age of 39.8 years and a mean maximum tumor diameter of 7.2 cm.The tumors were mostly located in the thigh,buttock,head and neck.The main clinical manifestations were masses(39.4%)and pain with swelling(12.1%).Soft tissue masses of equal or slightly low density were detected through CT examination,and there was no calcification or ossification in the lesion.Mainly low signal or mixed iso-hypo signal in T1WI and mostly mixed signal in T2WI(95.2%)were detected through MRI,and the enhancement was heterogeneous.The risk of recurrence or metastasis was 17.6%in 17 patients who were followed-up.ConclusionExtra-articular TSGCT within soft tissue is very rare.Diagnosis of TSGCT should be considered,if there were soft tissue masses from lower extremities,head and neck.Mixed signals were observed on MRI,and extra-articular sources were observed on CT.Surgical treatment can effectively control the progress of TSGCT.